Medicines for Progressive supranuclear palsy

What it is

Progressive supranuclear palsy (PSP) is a rare, progressive neurological condition in which certain areas of the brain gradually deteriorate over time, affecting balance, movement, eye movements, speech, swallowing, and, often, thinking. Like corticobasal degeneration, it is related to a build-up of an abnormal protein in the brain. PSP usually begins in later adult life and progresses over years. Its features can include: problems with balance and walking, often with a tendency to fall (frequently backwards), which is often an early and prominent feature; stiffness and slowness of movement (which can resemble Parkinson’s disease); a characteristic difficulty with eye movements — particularly difficulty moving the eyes up and down (looking down can become especially difficult), which affects activities such as reading, eating, and going down stairs, and is an important distinguishing feature; changes in speech (which can become slurred) and, over time, difficulty swallowing; changes in facial expression; and, often, changes in thinking, behaviour, and mood. As it progresses, PSP increasingly affects mobility (with a high risk of falls), communication, swallowing, and daily functioning, and people need increasing support. PSP can be difficult to diagnose, particularly early on, as its features (especially early) can overlap with Parkinson’s disease and other conditions; the characteristic eye-movement problems and the early, prominent balance problems and falls help distinguish it, and diagnosis is made by specialists based on the pattern of symptoms, examination, and tests. There is currently no cure for PSP, and no treatment that stops its progression, but care focuses on managing the symptoms, maintaining function and safety (particularly reducing the risk and impact of falls), maintaining quality of life for as long as possible, and supporting the person and family — through a specialist multidisciplinary team and, as the condition advances, supportive and palliative care. The key messages are that PSP is a rare, progressive brain condition affecting balance, movement, eye movements, and other functions, that falls are an important early feature, that there is no cure, and that a specialist team manages symptoms and supports the person and family.

How it is treated

Progressive supranuclear palsy is managed by a specialist multidisciplinary team, focusing on managing symptoms, maintaining function and safety (especially reducing falls), maintaining quality of life, and supporting the person and family; there is no cure or treatment to stop its progression, so care is supportive and individualised. Because PSP is rare and can overlap with other conditions early on, diagnosis is made by specialists (neurologists) based on the pattern of symptoms — particularly the early, prominent balance problems and falls, and the characteristic eye-movement difficulties — along with examination and tests (such as brain scans) to support the diagnosis and exclude other causes; diagnosis can be difficult, especially early. Once diagnosed, care focuses on managing the symptoms and supporting the person over time, and is multidisciplinary and tailored. Because falls are a major and early problem in PSP, reducing the risk and impact of falls is a particular priority — this includes physiotherapy (for balance, mobility, and strategies to reduce falls), aids and adaptations, home safety measures, and support with mobility, to help keep the person as safe and mobile as possible. Other aspects of care include: physiotherapy and occupational therapy to maintain movement, function, and daily living and provide aids and adaptations; speech and language therapy to help with speech and communication and, importantly, with swallowing (which is affected as the condition progresses, and needs careful management to maintain safe eating and nutrition); management of the eye-movement problems and their impact (with strategies and aids); and management of other symptoms — for example, medicines may be tried for some movement symptoms (though they are often less effective than in Parkinson’s disease), and treatments for other symptoms (such as mood or others) as needed. Managing the cognitive and behavioural changes, and supporting communication and daily life, are part of care. As PSP progresses and needs increase, care includes increasing support with all these areas, and supportive and palliative care focused on comfort and quality of life becomes important in the later stages, including careful management of swallowing and nutrition. Support for the person and, importantly, for family and carers — practical, emotional, and social — is central, and support organisations (such as PSP associations) provide information, support, and community. Advance care planning, made with the person while they are able, helps ensure care reflects their wishes. The reassuring messages, within the seriousness of this progressive condition, are that a specialist multidisciplinary team manages the symptoms, works to reduce falls and maintain function, safety, and quality of life for as long as possible, and supports the person and family throughout, and that support and, in the later stages, palliative care focus on comfort and wellbeing; so specialist multidisciplinary care, falls prevention, symptom management, and support for the person and family are central to managing PSP.